Gastrointest Radiol 17:31-33 0992)
Gastrointestinal
Radiology 9 Springer-Verlag New York Inc. 1992
Congenital Absence of the Portal Vein Yujiro Matsuoka, j K u n i Ohtomo, j Toshiyuki Okubo, l Junichi Nishikawa, ~Tetsuya Mine, 2 and Shinya Ohno 3 Department of Radiology and -~Fourth Department of Internal Medicine, Tokyo University Faculty of Medicine, Tokyo: and 30hno Hospital, Tokyo, Japan
Abstract. A b s e n c e o f the t e r m i n a l p o r t i o n of the portal v e i n a n d its i n t r a h e p a t i c b r a n c h e s was d e m o n s t r a t e d in a 2 2 - y e a r - o l d w o m a n with focal n o d u l a r h y p e r p l a s i a ( F N H ) of the liver. V a r i o u s imaging s t u d i e s a n d a n g i o g r a p h y s h o w e d that the p o r t a l v e i n e n t e r e d i n t o the i n f e r i o r v e n a c a v a j u s t a b o v e the r e n a l v e i n s . T h e clinical a n d radiological f e a t u r e s of this p a t i e n t a n d n i n e p r e v i o u s l y r e p o r t e d cases of this e n t i t y are h e r e i n r e v i e w e d . Key words: A b d o m e n , a n o m a l i e s - - P o r t a l v e i n , congenital a b s e n c e - - L i v e r , vascular supply
(CT) and ultrasound (US) of the abdomen showed a mass in the right hepatic lobe, fibroid uterus, and a very short portal vein entering into the suprarenal segment of IVC (Figs. 1 and 2). The intrahepatic branches of the portal vein were also absent. Hepatic arteriography disclosed a hypervascular tumor in the right lobe of the liver. Superior mesenteric arterial portography demonstrated that the superior mesenteric vein (SMV) entered the suprarenal IVC directly (Fig. 3). The SMV, inferior mesenteric vein (IMV), and splenic vein (SV) united normally. A catheter could be inserted into the visceral veins through the IVC (Fig. 4). Biopsy specimen obtained from the right lobe of the liver showed no intrahepatic portal venules in the normal liver, and the mass was FNH pathologically. The pelvic mass was resected, and it was leiomyoma of the uterus.
Discussion C o n g e n i t a l a b s e n c e o f the portal v e i n ( C A P V ) with v i s c e r a l v e n o u s r e t u r n to the i n f e r i o r v e n a c a v a (IVC) is a n e x t r e m e l y rare m a l f o r m a t i o n . O n l y n i n e c a s e s of this e n t i t y h a v e b e e n d e s c r i b e d [1-9]. W e r e c e n t l y e n c o u n t e r e d a 22-year-old w o m a n with focal n o d u l a r h y p e r p l a s i a ( F N H ) o f the liver w h o had CAPV.
D e v e l o p m e n t o f the portal v e i n o c c u r s in the 5- to 10-week e m b r y o . T h e portal v e i n is f o r m e d b y selec-
Case Report A 22-year-old woman was admitted to our hospital because of easy fatigability, hepatomegaly, and a palpable mass in her pelvis. The spine was scoliotic. Total bilirubin was 0.3 mg/dl (normal, 0.3-1.0); serum glutamic oxaloacetic transaminase (GOT) 70 IU/ L (normal, 9-31); serum glutamic pyruvic transaminase (GPT) 56 IU/L (normal, 4-30); albumin 3.8 g/dl (normal, 3.9-4.8); ammonia 78 tzg/dl (normal, 30-86); a-fetoprotein 5.14 ng/ml (normal,
Gastrointestinal
Radiology 9 Springer-Verlag New York Inc. 1992
Congenital Absence of the Portal Vein Yujiro Matsuoka, j K u n i Ohtomo, j Toshiyuki Okubo, l Junichi Nishikawa, ~Tetsuya Mine, 2 and Shinya Ohno 3 Department of Radiology and -~Fourth Department of Internal Medicine, Tokyo University Faculty of Medicine, Tokyo: and 30hno Hospital, Tokyo, Japan
Abstract. A b s e n c e o f the t e r m i n a l p o r t i o n of the portal v e i n a n d its i n t r a h e p a t i c b r a n c h e s was d e m o n s t r a t e d in a 2 2 - y e a r - o l d w o m a n with focal n o d u l a r h y p e r p l a s i a ( F N H ) of the liver. V a r i o u s imaging s t u d i e s a n d a n g i o g r a p h y s h o w e d that the p o r t a l v e i n e n t e r e d i n t o the i n f e r i o r v e n a c a v a j u s t a b o v e the r e n a l v e i n s . T h e clinical a n d radiological f e a t u r e s of this p a t i e n t a n d n i n e p r e v i o u s l y r e p o r t e d cases of this e n t i t y are h e r e i n r e v i e w e d . Key words: A b d o m e n , a n o m a l i e s - - P o r t a l v e i n , congenital a b s e n c e - - L i v e r , vascular supply
(CT) and ultrasound (US) of the abdomen showed a mass in the right hepatic lobe, fibroid uterus, and a very short portal vein entering into the suprarenal segment of IVC (Figs. 1 and 2). The intrahepatic branches of the portal vein were also absent. Hepatic arteriography disclosed a hypervascular tumor in the right lobe of the liver. Superior mesenteric arterial portography demonstrated that the superior mesenteric vein (SMV) entered the suprarenal IVC directly (Fig. 3). The SMV, inferior mesenteric vein (IMV), and splenic vein (SV) united normally. A catheter could be inserted into the visceral veins through the IVC (Fig. 4). Biopsy specimen obtained from the right lobe of the liver showed no intrahepatic portal venules in the normal liver, and the mass was FNH pathologically. The pelvic mass was resected, and it was leiomyoma of the uterus.
Discussion C o n g e n i t a l a b s e n c e o f the portal v e i n ( C A P V ) with v i s c e r a l v e n o u s r e t u r n to the i n f e r i o r v e n a c a v a (IVC) is a n e x t r e m e l y rare m a l f o r m a t i o n . O n l y n i n e c a s e s of this e n t i t y h a v e b e e n d e s c r i b e d [1-9]. W e r e c e n t l y e n c o u n t e r e d a 22-year-old w o m a n with focal n o d u l a r h y p e r p l a s i a ( F N H ) o f the liver w h o had CAPV.
D e v e l o p m e n t o f the portal v e i n o c c u r s in the 5- to 10-week e m b r y o . T h e portal v e i n is f o r m e d b y selec-
Case Report A 22-year-old woman was admitted to our hospital because of easy fatigability, hepatomegaly, and a palpable mass in her pelvis. The spine was scoliotic. Total bilirubin was 0.3 mg/dl (normal, 0.3-1.0); serum glutamic oxaloacetic transaminase (GOT) 70 IU/ L (normal, 9-31); serum glutamic pyruvic transaminase (GPT) 56 IU/L (normal, 4-30); albumin 3.8 g/dl (normal, 3.9-4.8); ammonia 78 tzg/dl (normal, 30-86); a-fetoprotein 5.14 ng/ml (normal,
